Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV002315814 | SCV000663271 | uncertain significance | Hereditary cancer-predisposing syndrome; Cardiovascular phenotype | 2020-03-18 | criteria provided, single submitter | clinical testing | The p.D2056V variant (also known as c.6167A>T), located in coding exon 41 of the NF1 gene, results from an A to T substitution at nucleotide position 6167. The aspartic acid at codon 2056 is replaced by valine, an amino acid with highly dissimilar properties. This amino acid position is highly conserved in available vertebrate species. In addition, the in silico prediction for this alteration is inconclusive. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |
Labcorp Genetics |
RCV000707050 | SCV000836130 | uncertain significance | Neurofibromatosis, type 1 | 2018-05-30 | criteria provided, single submitter | clinical testing | In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. Algorithms developed to predict the effect of missense changes on protein structure and function are either unavailable or do not agree on the potential impact of this missense change (SIFT: "Deleterious"; PolyPhen-2: "Probably Damaging"; Align-GVGD: "Class C0"). This variant has not been reported in the literature in individuals with NF1-related disease. ClinVar contains an entry for this variant (Variation ID: 480225). This variant is not present in population databases (ExAC no frequency). This sequence change replaces aspartic acid with valine at codon 2056 of the NF1 protein (p.Asp2056Val). The aspartic acid residue is moderately conserved and there is a large physicochemical difference between aspartic acid and valine. |
Genome- |
RCV000707050 | SCV002560944 | uncertain significance | Neurofibromatosis, type 1 | 2022-03-15 | criteria provided, single submitter | clinical testing |